Immune Mediated Diseases

Question 1

Tipoffs for vasculitis

Answer 1

• mononeutritis multiplex
• palpable purpura
• “can’t tell why they are sick”

Question 2

Polyarteritis Nodosa

Answer 2

necrotizing vasculitis

• Etiologies: hep b/c, cryoglobulins
• Patchy inflammation of all 3 layers of large arteries – true aneurysms (undergo thrombosis)
• gangrene from ischemia
• ANCA-negative
• fibrinoid deposits w/ elastic damage
• negative hematuria

Question 3

Microscopic Polyangiitis

Answer 3

Positive ANCA, most likely involving capillaries

- hematuria

Question 4

ANCA responsible for microscopic polyangiitis

Answer 4

p-ANCA (anti-MPO)

Question 5

Wegener’s Granulomatosis

Answer 5

• positive ANCA
• granulomas and patchy necrosis of a/v
• necrosis above neck, lungs, and kidneys
• episcleritis, saddle nose (necrosis of nasal septum)

Question 6

ANCA responsible for Wegener’s Granulomatosis

Answer 6

c-ANCA (PR3-ANCA)

Question 7

Kawasaki

Answer 7

damage to coronary arteries

Question 8

Henoch-Schonlein

Answer 8

purpura and IgA nephropathy

- arthritis, GI bleeding

Question 9

Cyroglobulinemia

Answer 9

marginally soluble protein precipitate in cool places or where blood is concentrated
(hep C Ab/Ag, rheumatoid factors)

Question 10

Churg-Strauss

Answer 10

“allergic granulomatosis”

• abundant eosinophils
• caused by LT antagonist Rx for asthma
• mononeuritis multiplex is common
• occulsion of heart microvasculature causes death

Question 11

Behcet’s Disease

Answer 11

AI against HSP

• mouth lesions(HLA-B51)
• eye lesions (hypopyon in lower eyelid)
• genital, skin lesions
• pathergy test positive

Question 12

What is the pathergy test for?

Answer 12

Behcet’s

- ulcer will form at site of sterile needle prick

Question 13

What is amyloid?

Answer 13

proteins that accumulate in beta-pleated form

• effects many organs
• can lead to carpal tunnel

Question 14

How does amyloid stain?

Answer 14

congo red with apple green birefringence

Question 15

Amyloid AA

Answer 15

serum amyloid associated protein

- prolonged inflammation w/ macrophages, osteomyelitis, TB, leprosy, rheumatoid arthritis

Question 16

Amyloid AL

Answer 16

immunoglobulin light chains

- plasma cell neoplasia

Question 17

Amyloid ATTR (AF)

Answer 17

transthyretin

• may get liver transplant
• carries vitamin A

Question 18

Amyloid b2m (AH)

Answer 18

beta-two microglobulin

• HLA light chains
• long standing kidney failure

Question 19

What amyloid causes restrictive cardiomyopathy (stiff heart)?

Answer 19

Amyloid AL

Question 20

What do patients with defective innate immunity have difficulty with?

Answer 20

bacteria

Question 21

Chediak-Higashi disease

Answer 21

defect in phagolysosome function

• also interferes with melanosome formation
• causes neuropathy

Question 22

What is the defect mechanism in Chronic Granulomatous Diseases?

Answer 22

oxygen-dependent bacterial killing

- require macrophage system to handle bacteria

Question 23

What do patients with complement component deficiencies have trouble fighting?

Answer 23

Bacteria

Question 24

What disease do C2/C4/C1q deficiencies mimic?

Answer 24

Lupus

- alternate pathway works to handle bacteria

Question 25

What problems come from a C3 deficiency?

Answer 25

bacteria and with immune complex membranoproliferative glomerulonephritis

Question 26

What problems come from a C5-C9 deficiency?

Answer 26

Difficulty with bacteria | - most importantly neisseria

Question 27

What defect causes hereditary angioedema?

Answer 27

C1-esterase inhibitor

Question 28

Deficiency in T-cells can lead to increased risk for --

Answer 28

candida, CMV, pneumocystis

Question 29

Deficiency in B-cells can lead to increased risk for --

Answer 29

bacterial infections (once mom's Ab clear)

Question 30

Severe Combined Immunodeficiency

Answer 30

Thymus produces few T-cells | - X-linked is most common (mutation of IL2RG -- g-chain of IL)

Question 31

Bruton's disease

Answer 31

X-linked agammaglobulinemia - lack of bruton's tyrosine kinsase (B-cells do not mature) - trouble with bacteria

Question 32

DiGeorge Syndrome

Answer 32

"thymic dysembryogenesis" - lack parathyroids, thymus - have midline cardiac defects

Question 33

Hyper-IgM

Answer 33

patients can't make IgG, IgA, IgE - mutation of CD40 on X-Chr - low neutrophils/platelets - pneumocystis infections

Question 34

Isolated IgA deficiency

Answer 34

Anaphylaxis occurs from donor IgA | - seen at second blood transfusion

Question 35

Sex-Linked Lymphoproliferative syndrome

Answer 35

Mutant SH2D1A -- signal transduction protein - poor response to viruses allows Epstein-Barr virus to produce over-growth of lymphocytes - destroy liver/marrow

Question 36

Wiskott-Aldrich

Answer 36

X-linked, tiny platelets, eczema, deficient T-cells, WASp locus, ataxia, telangiectasia

Question 37

Hyper-IgE

Answer 37

"Job's syndrome" - too much IgE, faulty neutrophil movement - Staph causes problems